title: Wilms' tumour with persistent intravascular extension: a review of the surgical aspects of management.
abstract: OBJECTIVE
  To review the surgical management of Wilms' tumour with persistent intravascular (vena caval +/- atrial) tumour extension.
  
  
  METHODS
  Data were collected regarding operative details, tumour and 'thrombus' histology, and long-term outcome for patients with Wilms' tumour with cavo-artial extension.
  
  
  RESULTS
  From 1988 to 2004, 13 patients underwent treatment for Wilms' tumour with persistent intravascular extension. Preoperative chemotherapy was administered in 11/13 patients and postoperative radiotherapy in eight patients. Intravascular involvement was upto IVC (5), and right atrium (8) patients. Techniques employed for excision of intra-vascular component were: local cavotomy (3), extensive infra-diaphragmatic cavotomy without cardiopulmonary bypass (CPB) (1), and excision of cavo-atrial tumour with CPB (+/- hypothermia and cardiac arrest) (9). Mean time on CPB was 90 min. Caval repair was accomplished by primary repair (6) and pericardial graft in (7) patients. There were no intraoperative deaths and few major complications. Tumour thrombus contained malignant cells in 10/13 cases. Mean follow up has been for 55.4 months. To date, seven patients remain disease-free (one lost to follow up), disease recurred in five patients, three of whom have died. There were no symptoms related to the graft.
  
  
  CONCLUSIONS
  Surgery for Wilms' tumour with persistent intravascular extension despite chemotherapy is technically challenging. CPB +/- hypothermia and cardiac arrest and extensive caval repair with a graft is safe and reliable in the long term.
authors.affiliation: Department of Paediatric Urology, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK. murthigvs@doctors.org.uk
authors.forename: G V
authors.initials: GV
authors.lastname: Murthi
authors.forename: E
authors.initials: E
authors.lastname: Kocyildirim
authors.forename: S
authors.initials: S
authors.lastname: Sellathury
authors.forename: P M
authors.initials: PM
authors.lastname: Cuckow
authors.forename: D T
authors.initials: DT
authors.lastname: Wilcox
authors.forename: A
authors.initials: A
authors.lastname: Michalski
authors.forename: N J
authors.initials: NJ
authors.lastname: Sebire
authors.forename: M J
authors.initials: MJ
authors.lastname: Elliott
authors.forename: P G
authors.initials: PG
authors.lastname: Duffy